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[Neurological catastrophes due to medical errors]. / Catástrofes neurológicas por desconocimiento y retraso diagnóstico.

In 1999, the American Institute of Medicine reported an enormous rate of medical errors, representing the fifth cause of death. In Spain, there is no reliable information on the number and nature of medical adverse events, but the situation is probably similar to that described in the USA in 1999, if not higher. Diagnostic errors account for more than half of neurological adverse events and these errors can be catastrophic if the natural progression of the neurological disorder causes severe sequels or even death when the patient is left untreated. To improve patient safety, research must be undertaken to determine how these errors are produced and to develop strategies to prevent inappropriate conduct. Among many other elements, it is important to create teamwork, improve neurological knowledge among general practitioners and residents, to design clinical practice guidelines aimed at patient safety, and to promote policies that reward the absence of errors. In general, medical errors are neither exclusively due to lack of experience nor to insufficient medical knowledge, but rather to faulty organization of medical care. Therefore, it is preferable to monitor healthcare organization rather than to blame the individual supposedly responsible for the error.

Catástrofes neurológicas por desconocimiento y retraso diagnóstico / Neurological catastrophes due to medical errors

El Instituto Americano de Medicina desveló en 1999 que en Estados Unidos se producía una enorme cantidad de errores médicos; de hecho, eran la quinta causa de muerte en este país. Aunque no se sabe si en España se produce el mismo número de errores, existen razones para pensar que el problema es similar, si no mayor. Los errores diagnósticos representan más de la mitad del total de efectos adversos, que pueden ser catastróficos —o tener consecuencias catastróficas— si el proceso neurológico, dejado a su evolución natural, causa la muerte o secuelas severas, es decir, el error puede tener consecuencias catastróficas si no se actúa adecuadamente para interrumpir esta evolución. Por ello es necesario investigar cómo se producen los errores y diseñar estrategias para evitarlos y prevenir conductas inadecuadas. Entre otras, es importante organizar equipos de trabajo, mejorar el conocimiento de la neurología entre los médicos generales y residentes que asisten a este tipo de enfermo, elaborar guías orientadas a salvaguardar la seguridad del paciente y promover políticas adecuadas que premien la falta de errores. Los errores médicos no se deben, por lo general, al desconocimiento o a la falta de experiencia; en ellos influye de forma decisiva una organización asistencial inadecuada. Por este motivo será preferible vigilar la organización de la asistencia y no descargar toda la culpa a nivel individual (AU)

In 1999, the American Institute of Medicine reported an enormous rate of medical errors, representing the fifth cause of death. In Spain, there is no reliable information on the number and nature of medical adverse events, but the situation is probably similar to that described in the USA in 1999, if not higher. Diagnostic errors account for more than half of neurological adverse events and these errors can be catastrophic if the natural progression of the neurological disorder causes severe sequels or even death when the patient is left untreated. To improve patient safety, research must be undertaken to determine how these errors are produced and to develop strategies to prevent inappropriate conduct. Among many other elements, it is important to create teamwork, improve neurological knowledge among general practitioners and residents, to design clinical practice guidelines aimed at patient safety, and to promote policies that reward the absence of errors. In general, medical errors are neither exclusively due to lack of experience nor to insufficient medical knowledge, but rather to faulty organization of medical care. Therefore, it is preferable to monitor healthcare organization rather than to blame the individual supposedly responsible for the error (AU)

Apolipoproteína e4 en la demencia con cuerpos de Lewy / Apolipoprotein e4 in dementia with Lewy bodies

Introducci¨®n. Existe una fuerte asociaci¨®n entre el alelo¦Å4 de la apolipoprote¨ªna E (APOE) y enfermedad de Alzheimer(EA), constituyendo este alelo un factor de riesgopara padecer esta enfermedad. Su asociaci¨®n con la demenciacon cuerpos de Lewy (DCL) es objeto de controversia. Enla DCL la presencia de APOE4 se ha relacionado con unamayor carga de placas seniles y degeneraci¨®n neurofibrilar.M¨¦todo. Estudio de casos y controles en el que se determin¨®el genotipo APOE mediante la t¨¦cnica de reacci¨®nen cadena de la polimerasa (PCR) modificada de Wenhamen 306 pacientes diagnosticados de EA probable, criteriosNINCDS-ADRDA, 58 casos de DCL probable, criterios de consensode McKeith et al. (1996), todos ellos con SPECT con 123IFP-CIT patol¨®gico, y 80 controles normales (CN) de edad y distribuci¨®npor sexos similares.Resultados. La frecuencia de alelos fue la siguiente:DCL ¦Å4: 16%; ¦Å3: 75%; ¦Å2: 9%; EA ¦Å4: 32%; ¦Å3: 67%; ¦Å2:1%; CN ¦Å4: 12%; ¦Å3: 83%; ¦Å2: 5%. En los tres grupos la distribuci¨®nde alelos en ambos sexos fue similar.Conclusiones. En la DCL la frecuencia de ¦Å4 (16%) esmuy inferior a la de la EA (32%) y muy pr¨®xima a la cifra delos CN (12%). Teniendo en cuenta que la presencia de alteracionesmorfopatol¨®gicas tipo Alzheimer en la DCL, fundamentalmentedegeneraci¨®n neurofibrilar, se correlacionainversamente con la presencia de signos parkinsonianos, esposible que este grupo represente a las formas puras de laenfermedad, aunque la falta de comprobaci¨®n neuropatol¨®gicano permite confirmar esta hip¨®tesis (AU)

Introduction. There is a strong association betweenthe ¦Å4 allele of apolipoprotein E (APOE) and Alzheimer¡¯sdisease (AD). This converts this allele into a risk factorfor the development of AD. The association between APOE4and dementia with Lewy bodies (DLB) is under discussion.In DLB, the presence of APOE4 has been related with a greateramount of senile plaques and neurofibrillary tangles.Method. This is a case-control study in which the APOEgenotype was determined using the modified PCR techniqueof Wenham in 306 patients with diagnosis of probably AD,NINCDS-ADRDA criteria, 58 cases of probably DLB, McKeithet al. consensus criteria (1996), all of them with SPECT withpathological 123I-FP-CIT and 80 normal controls (NC) havingsimilar age and gender distribution.Results. The frequency of alleles was: DLB group ¦Å4:16%; ¦Å3: 75%; ¦Å2: 9%; AD: ¦Å4: 32%; ¦Å3: 67%; ¦Å2: 1%;and in the normal control group: ¦Å4: 12%; ¦Å3: 83%; ¦Å2:5%. The percentage of alleles in both genders was similarin the three groups.Conclusions. APOE4 percentage in DLB group (16%)was lower than in AD group (32 %), and similar to thecontrol group (12 %). Considering that the presence ofmorphopathological Alzheimer type alterations in DBL,essentially neurofibrillary tangles, is inversely correlatedwith the presence of Parkinsonian signs, this group mayrepresent pure forms of the disease, although the lack ofneuropathological demonstration does not make it possibleto confirm this hypothesis (AU)

[Apolipoprotein E4 in dementia with Lewy bodies]. / Apolipoproteína e4 en la demencia con cuerpos de Lewy.

INTRODUCTION: There is a strong association between the e4 allele of apolipoprotein E (APOE) and Alzheimer's disease (AD). This converts this allele into a risk factor for the development of AD. The association between APOE4 and dementia with Lewy bodies (DLB) is under discussion. In DLB, the presence of APOE4 has been related with a greater amount of senile plaques and neurofibrillary tangles. METHOD: This is a case-control study in which the APOE genotype was determined using the modified PCR technique of Wenham in 306 patients with diagnosis of probably AD, NINCDS-ADRDA criteria, 58 cases of probably DLB, McKeith et al. consensus criteria (1996), all of them with SPECT with pathological 123I-FP-CIT and 80 normal controls (NC) having similar age and gender distribution. RESULTS: The frequency of alleles was: DLB group epsilon4: 16%; epsilon3: 75%; epsilon2: 9%; AD: epsilon4: 32%; epsilon3: 67%; epsilon2: 1%; and in the normal control group: epsilon4: 12%; epsilon3: 83%; epsilon2: 5%. The percentage of alleles in both genders was similar in the three groups. CONCLUSIONS: APOE4 percentage in DLB group (16%) was lower than in AD group (32%), and similar to the control group (12%). Considering that the presence of morphopathological Alzheimer type alterations in DBL, essentially neurofibrillary tangles, is inversely correlated with the presence of Parkinsonian signs, this group may represent pure forms of the disease, although the lack of neuropathological demonstration does not make it possible to confirm this hypothesis.

Sintomatología miccional y alteraciones urodinámicas en la enfermedad de Parkinson / Urinary symptoms and urodynamic findings in Parkinson's disease

Introducción. Los síntomas urinarios en la enfermedad de Parkinson (EP) son síntomas menores, pero invalidantes. Analizamos su correlación con síntomas motores, duración y gravedad de la EP y con las alteraciones urodinámicas observadas. También estudiamos la respuesta al tratamiento con tolterodina. Métodos. Se incluyeron pacientes con EP y puntuaciones en los cuestionarios de síntomas urinarios superiores a 7 en varones (IPSS) y a 5 en mujeres (IU-4). Se analizó intensidad de los síntomas neurológicos (escala UPDRS), gravedad de la EP (escala Hohen-Yahr), parámetros del estudio urodinámico y sedimento urinario. En varones además se realizó tacto rectal y ecografía abdominal para valorar patología prostática obstructiva. Los pacientes sin obstrucción al flujo urinario recibieron tratamiento con tolterodina. Resultados. Diecinueve pacientes fueron seleccionados, excluyéndose 3 por sedimento patológico. En los 16 restantes se observó una correlación positiva de la intensidad de los síntomas urinarios con los años de evolución de la EP (p<0,01) y con la rigidez (p<0,01), que fue el síntoma más intenso. En 13 casos (81,2 %) se detectó hiperactividad de detrusor, de los que 4 presentaron datos urodinámicos de obstrucción. El porcentaje de mejoría de los síntomas urinarios en los 9 casos tratados con tolterodina fue bajo (33 %). Conclusiones. La gravedad de los síntomas urinarios se correlaciona con el grado de rigidez y con los años de evolución de la EP. La combinación de los cuestionarios y el estudio urodinámico permiten identificar el tipo de disfunción vesical y seleccionar a los pacientes tributarios de tratamiento anticolinérgico. La tolterodina redujo los síntomas de urgencia y frecuencia miccional en la EP, pero fue ineficaz sobre la incontinencia urinaria

Introduction. Urinary symptoms in Parkinson's disease (PD) are minor but disabling. We have analyzed correlation of urinary symptoms with motor symptoms, duration and severity of PD and urodynamic abnormalities observed. Response to treatment with tolterodine was also assessed. Methods. PD male patients with a score in IPSS questionnaire over 7 and female patients with a score in IU-4 scale over 5 were included in the study. Intensity of neurological symptoms (UPDRS score), seriousness of PD (Hohen-Yahr stage), urodynamic parameters, and urinary sediment were analyzed in each patient. Abdominal ultrasonography and rectal examination were performed in males to exclude obstructive prosthatic pathology. Patients without evidence of urinary flow obstruction were treated with tolterodine. Results. Three out of the 19 patients were excluded because of abnormal urinary sediment and the rest (n = 16) were included. Urinary symptoms correlated with rigidity severity (p < 0.01) and years of evolution of PD (p<0.01). Rigidity (p<0.01) was the neurological sign with the highest UPDRS motor scale score. Overactive bladder was present in 13 cases (81.2 %) and 4 of them had urinary flow obstruction. Clinical improvement in nine patients treated with tolterodine was mild (33 %). Conclusions. Urinary symptoms correlate with rigidity severity and with years of evolution of PD. The use of both the urinary questionnaire and urodynamic study allow us to identify the type of bladder dysfunction and select the patients who would benefit the most from anticholinergics. Tolterodine reduced miccional urgency and frequency in PD, but was ineffective on urinary incontinence

[Urinary symptoms and urodynamic findings in Parkinson's disease]. / Sintomatología miccional y alteraciones urodinámicas en la enfermedad de Parkinson.

INTRODUCTION: Urinary symptoms in Parkinson's disease (PD) are minor but disabling. We have analyzed correlation of urinary symptoms with motor symptoms, duration and severity of PD and urodynamic abnormalities observed. Response to treatment with tolterodine was also assessed. METHODS: PD male patients with a score in IPSS questionnaire over 7 and female patients with a score in IU-4 scale over 5 were included in the study. Intensity of neurological symptoms (UPDRS score), seriousness of PD (Hohen-Yahr stage), urodynamic parameters, and urinary sediment were analyzed in each patient. Abdominal ultrasonography and rectal examination were performed in males to exclude obstructive prostatic pathology. Patients without evidence of urinary flow obstruction were treated with tolterodine. RESULTS: Three out of the 19 patients were excluded because of abnormal urinary sediment and the rest (n = 16) were included. Urinary symptoms correlated with rigidity severity (p < 0.01) and years of evolution of PD (p < 0.01). Rigidity (p < 0.01) was the neurological sign with the highest UPDRS motor scale score. Overactive bladder was present in 13 cases (81.2%) and 4 of them had urinary flow obstruction. Clinical improvement in nine patients treated with tolterodine was mild (33%). CONCLUSIONS: Urinary symptoms correlate with rigidity severity and with years of evolution of PD. The use of both the urinary questionnaire and urodynamic study allow us to identify the type of bladder dysfunction and select the patients who would benefit the most from anticholinergics. Tolterodine reduced miccional urgency and frequency in PD, but was ineffective on urinary incontinence.

[Advanced dementia in Alzheimer's disease and memantine]. / Demencia avanzada de la enfermedad de Alzheimer y memantina.

INTRODUCTION: Alzheimer's disease (AD) causes dementia with a progressive course. Until now, research has been aimed mainly at studying its early, pre-dementia and mild or mild to moderate dementia phases in an attempt to find treatments with which to cure it or at least halt its progression. It is true that the latest cognitive therapies are effective up to a point, but the fact is that many patients reach the state of advanced AD, which gives rise to a number of cognitive and behavioural disorders that entail all kinds of problems on a personal, familial and community health level. AIMS: This study reviews the progress of AD, the length of its stages, the speed at which the disease courses and patients' survival. Advanced AD is defined, its clinical features and the functional disability it causes are described, and we analyse the tests and scales that must be used to measure how the process is progressing and the effectiveness of the distinct forms of treatment used in the later phase of AD, which are different to those employed in the early stages. All this allows us to analyse the results of clinical trials carried out with memantine, an NMDA (N-methyl D-aspartate) receptor antagonist. We review its pharmacological characteristics and its use in everyday practice. Lastly, we refer to the classical symptomatic treatments that are usually employed to control the frequent and intense behavioural disorders produced in the advanced phase of AD. CONCLUSIONS: The later stage of AD requires our attention because many patients reach and remain for a long time in this phase, which leads to considerable personal and social disorders. Several scales and tests have been adapted to these later phases of AD, enabling the clinician to evaluate the patient, monitor the progress of the disease and determine the effectiveness of different treatments. Memantine, the most recent drug approved for use with this disease, has proved to be effective in the treatment of patients with advanced AD. This pharmaceutical has been added to the list of well-known classical medicines, such as neuroleptic drugs, antidepressants, anxiolytic agents and others, which can be used to diminish the behavioural disorders in these patients and improve their quality of life, as well as that of their caregivers.

Demencia avanzada de la enfermedad de Alzheimer y memantina / Advanced dementia in alzheimer's disease and memantine

Introducción. La enfermedad de Alzheimer (EA) causa una demencia de curso progresivo. Hasta ahora, la investigación se ha dirigido primordialmente al estudio de sus fases precoces, predemencial y de demencia leve o leve a moderada, con objeto de encontrar tratamientos para curarla o, al menos, detener su progresión. Es cierto que los nuevos tratamientos cognitivos son modestamente efectivos, pero la realidad es que numerosos pacientes llegan a padecer una EA avanzada, que provoca numerosas alteraciones cognitivas y conductuales que, a su vez, conllevan todo tipo de problemas personales, familiares y sociosanitarios. Objetivos. Este trabajo revisa la evolución de la EA, la duración de sus estadios, la velocidad del curso de la enfermedad y la supervivencia de los pacientes. A continuación, se define la EA avanzada, se describen sus características clínicas y la discapacidad funcional que causa, y se analizan los test y escalas que deben utilizarse para medir la progresión del proceso y la efectividad del tratamiento en la fase tardía de la EA, diferentes de los empleados en las fases precoces. Todo ello permite analizar los resultados de los ensayos clínicos realizados con memantina, un antagonista del receptor NMDA (N-metil-Daspartato), de la que se revisan sus características farmacológicas y su utilización en la práctica diaria. Finalmente, se refieren los tratamientos sintomáticos clásicos que se suelen utilizar en la fase avanzada de la EA para controlar los frecuentes e intensos trastornos conductuales que causa la enfermedad. Conclusiones. La fase tardía de la EA requiere nuestra atención, porque numerosos enfermos la alcanzan y permanecen en ella durante mucho tiempo, lo que ocasiona considerables trastornos personales y sociales. Ya existen escalas y test adaptados a estas fases tardías de la EA que permiten evaluar al paciente, controlar la evolución de la enfermedad y determinar la efectividad de los tratamientos. En este sentido, la memantina, el último medicamento aprobado para esta enfermedad, se ha mostrado eficaz para tratar los enfermos que padecen una EA avanzada. Este medicamento se ha añadido a los diversos medicamentos clásicos bien conocidos, como neurolépticos, antidepresivos, ansiolíticos y otros, con los que se puede conseguir disminuir el trastorno conductual de estos pacientes y mejorar su calidad de vida y la de sus cuidadores

Introduction. Alzheimer’s disease (AD) causes dementia with a progressive course. Until now, research has been aimed mainly at studying its early, pre-dementia and mild or mild to moderate dementia phases in an attempt to find treatments with which to cure it or at least halt its progression. It is true that the latest cognitive therapies are effective up to a point, but the fact is that many patients reach the state of advanced AD, which gives rise to a number of cognitive and behavioural disorders that entail all kinds of problems on a personal, familial and community health level. Aims. This study reviews the progress of AD, the length of its stages, the speed at which the disease courses and patients’ survival. Advanced AD is defined, its clinical features and the functional disability it causes are described, and we analyse the tests and scales that must be used to measure how the process is progressing and the effectiveness of the distinct forms of treatment used in the later phase of AD, which are different to those employed in the early stages. All this allows us to analyse the results of clinical trials carried out with memantine, an NMDA (N-methyl D-aspartate) receptor antagonist. We review its pharmacological characteristics and its use in everyday practice. Lastly, we refer to the classical symptomatic treatments that are usually employed to control the frequent and intense behavioural disorders produced in the advanced phase of AD. Conclusions. The later stage of AD requires our attention because many patients reach and remain for a long time in this phase, which leads to considerable personal and social disorders. Several scales and tests have been adapted to these later phases of AD, enabling the clinician to evaluate the patient, monitor the progress of the disease and determine the effectiveness of different treatments. Memantine, the most recent drug approved for use with this disease, has proved to be effective in the treatment of patients with advanced AD. This pharmaceutical has been added to the list of well-known classical medicines, such as neuroleptic drugs, antidepressants, anxiolytic agents and others, which can be used to diminish the behavioural disorders in these patients and improve their quality of life, as well as that of their caregivers

Prophylactic treatment of episodic cluster headache with intravenous bolus of methylprednisolone.

We evaluated the efficacy of intravenous boluses of methylprednisolone followed by prednisone as a prophylactic treatment for episodic cluster headache. Fourteen male patients (mean age, 42.54 years) with episodic cluster headache were treated with 250-mg boluses of methylprednisolone on 3 consecutive days, followed by prednisone (90 mg/day orally) with gradual tapering in four weeks. Headache parameters of the active phases treated with methylprednisolone were compared with those of previous active phases in the same patients treated with other prophylactic medications. The primary efficacy criterion was decrease in the frequency of attacks during the first month of treatment. The statistical differences were calculated using Wilcoxon's test. The attacks were significantly less frequent in the active phases treated with methylprednisolone boluses than those treated with other medications ( p<0.05). This treatment seems to be more effective than the usual prophylactic treatments for episodic cluster headache.

Transient topographical disorientation.

Transient topographical disorientation (TTD) is a short-lasting inability to find one's way in a familiar environment, while the patient remains conscious and is able to recall what happened. We report the study of 10 patients with episodes of TTD, studied on the days following the last episode. The episodes of TTD could be separated into two types: the patients either reported difficulties in spatial orientation with preserved abilities to recognize landmarks and objects, or the difficulties appeared with the recognition of landmarks. Tests exploring spatial orientation, as well as higher visuoperceptive capacities were altered in most of the patients and brain SPECT showed hypoperfusion of the right hemisphere in all patients, which could also be demonstrated 2 years later in some cases. Altogether, our findings suggest that TTD is frequently associated with a more persistent right hemisphere dysfunction of unknown cause. This chronic alteration could represent either a sequel of the acute episode or a preexisting right hemisphere deficit, which inclined the acute insult to be manifested as TTD.

[Alzheimer's disease and women]. / Enfermedad de Alzheimer y mujer.

INTRODUCTION AND DEVELOPMENT: This work reviews the relation between Alzheimer s disease (AD) and women, a very interesting issue both for its socio economic, and etiopathogenic and therapeutic aspects. Much of the prevalent research conducted in this field shows that a higher proportion of suffers from this disease are women, and in the work on incidence there is at least a tendency toward the same conclusion, especially at a very advanced age. In fact, the risk of suffering from AD is greater among women and most of the patients we attend are females, which is to a large extent associated with the fact that women live longer. However, it is possible that there are other biological factors involved and for this reason the action of estrogens on the brain and the consequences of women s being deprived of them during menopause is of special interest. CONCLUSIONS: Different studies have shown that the administration of hormone replacement therapy (HRT) lowers the risk of suffering from this disease, although design defects make it necessary to wait for the conclusions from other research work currently being conducted. There are also data that supports the idea that HRT can be beneficial in AD if it is administered in suitable doses. Obviously gender can influence or modulate other risk factors (RF). Genetic factors are not easily modified and for this reason research is currently aimed at factors in which a strong environmental component is involved. Another very controversial possible RF is lack of schooling, but some data support the notion that its influence can be especially harmful among females. This is a very important hypothesis because women make up the greater part of the illiterate population in Spain. Finally, women are also prevalent among caregivers and, therefore, suffer AD from both angles: they must care and be cared for. The reaction to this situation seems to be gender specific, which means that women in particular suffer the consequences of the lack of reciprocity brought about by AD something that does not happen in other equally devastating chronic processes, but which affect the physical sphere.

Enfermedad de Alzheimer y mujer / Alzheimer's disease and women

Introducción y desarrollo. Este trabajo revisa la relación entre la enfermedad de Alzheimer (EA) y la mujer, un capítulo muy interesante, tanto por sus aspectos socioeconómicos, como por los etiopatogénicos y terapéuticos. En efecto, gran parte de los trabajos de prevalencia muestran un predominio femenino en esta enfermedad, y en los trabajos de incidencia hay al menos una tendencia al predominio femenino, especialmente en edad muy avanzada. De hecho, el riesgo de padecer una EA es superior en el sexo femenino y la mayor parte de los enfermos que asistimos son mujeres, lo que se relaciona en gran medida con la mayor supervivencia de la mujer. No obstante, es posible que intervengan otros factores biológicos y, en este sentido, la acción de los estrógenos sobre el cerebro y las consecuencias de su deprivación durante la menopausia son muy interesantes. Conclusiones. Diversos estudios han mostrado que la administración de terapéutica hormonal sustitutiva (THS) disminuye el riesgo de padecer la enfermedad, aunque defectos de diseño hacen necesario esperar las conclusiones de otros trabajos en marcha. También existen datos que apoyan que la THS puede conseguir beneficios en la EA si se administra a dosis adecuadas. Por supuesto, el sexo puede influir o modular otros factores de riesgo (FR). Los factores genéticos se modifican poco y, por ello, se prefiere de momento dirigir la atención a los factores de fuerte componente ambiental. En este sentido, la falta de escolarización es un posible FR muy discutido, pero ciertos datos apoyan que su influencia puede ser especialmente perniciosa en el sexo femenino; este es un dato importante, porque precisamente entre los analfabetos de nuestro país predominan las mujeres. Finalmente, en lo que se refiere a los cuidadores, también aquí predominan las mujeres, que, por tanto, sufren la EA en ambas vertientes: recibir y administrar cuidados. La reacción a esta situación parece ser genero específica, de forma que la mujer sufre especialmente las consecuencias de la falta de reciprocidad que ocasiona la EA, algo que no ocurre en otros procesos crónicos igualmente devastadores, pero que afectan la esfera física (AU)

Statuslike SUNCT in two young women.

OBJECTIVE: To describe the statuslike pattern of SUNCT (short-lasting unilateral neuralgiform pain with conjunctival injection and tearing) in two young women. BACKGROUND: SUNCT syndrome is a rare condition characterized by a short-lasting periocular pain associated with marked autonomic symptoms. Twenty-five cases have been reported in the literature with a high preponderance of males and a mean age of 51 years. The frequency of episodes shows a wide variability, not just among individuals but also in the same patient, and a statuslike pattern of almost continuous attacks has been described. METHODS: We report the cases of two young women (aged 26 and 23 years) with typical SUNCT features who suffered bouts of up to 60 paroxysms of pain per hour. Paraclinical investigations showed no abnormalities. CONCLUSIONS: Although unusual, paroxysms in SUNCT may overlap into a clinical status. A strong relationship with hormonal changes was noted in one patient. In both cases, the pain was refractory to treatment with indomethacin, carbamazepine, and hypnotics, and only intravenous methylprednisolone with oral carbamazepine may have been partially effective in one case.

[Posterior cortical atrophy]. / Atrofia cortical posterior.

INTRODUCTION: Posterior cortical atrophy (PCA) is a dementing syndrome characterized by an early alteration of higher visual functions. Patients usually develop a perceptive visual agnosia related to Balint syndrome. DEVELOPMENT: Verification showed Alzheimer s disease (AD) in most PCA observations (13/14 cases), and it can be concluded that there is a posterior variant of AD with prominent visual symptomatology. However, most PCA cases have not been verified and the nature of the disorder remains unknown in these observations. An early and severe unilateral occipital horn dilatation was found in one out of every six cases with non verified PCA. To the best of our knowledge, this image has not been described in AD and these observations could be of different nature. On the other hand, some non verified PCA cases have occasionally manifested as an associative visual agnosia. The nature of this type of associative disorder is unknown, but it could be similar to the nature of semantic dementia, a non Alzheimer s syndrome related to unspecific lesions of temporal lobes.

Atrofia cortical posterior / Posterior cortical atrophy

Introducción. La atrofia cortical posterior (ACP) es un síndrome clínico de curso crónico, caracterizado por una afectación precoz e intensa de las funciones visuales superiores, con conservación inicial de otras funciones cognitivas. Los enfermos desarrollan agnosia visual, generalmente aperceptiva, relacionada con síndrome de Balint, a lo que finalmente se añade demencia. Desarrollo. La revisión de los casos verificados demuestra que la ACP se debe generalmente (13/14 observaciones) a enfermedad de Alzheimer (EA), de forma que esta entidad podría considerarse una variedad posterior de EA. Sin embargo, la mayoría de las observaciones de ACP no están verificadas y se desconoce su naturaleza. Aproximadamente en uno de cada seis enfermos con ACP no verificada existe una dilatación unilateral, precoz y focal, del asta occipital, algo no descrito en la EA. Por otra parte, se han descrito casos no verificados en los que la ACP causa agnosia visual asociativa. La naturaleza de este tipo de ACP podría ser diferente de la EA y similar a la de la demencia semántica, un proceso degenerativo inespecífico relacionado con la alteración de estructuras temporales (AU)

[Psychotic symptoms and Alzheimer's disease]. / Síntomas psicóticos y enfermedad de Alzheimer.

BACKGROUND: Psychotic symptoms appear during the course of Alzheimer's disease, but their frequency and intensity vary according to different studies and their nature remains unsettled. OBJECTIVES: To study the frequency and intensity of psychotic symptoms in two transversal series of patients with Alzheimer's disease and analyze its relationship with the duration of the disease and severity of cognitive impairment. PATIENTS AND METHODS: This study has been carried out in patients suffering from probable Alzheimer's disease (NINDS-ADRDA criteria). The stage of the disease was determined according to FAST, and the intensity of cognitive impairment in Mini Mental State Examination was classified as mild, moderate or severe. Frequency and intensity of psychotic symptoms (delusions, hallucinations and misidentifications) were determined by means of semistructured interviews (BEHAVE-AD 78 patients and CUSPAD 69 patients). The results obtained in these three groups of patients were compared through ANOVA variance analysis and mean contrast. Variance and covariance analysis were done to determine the relationship between psychotic symptoms and other variables (degree of cognitive impairment, length of evolution and stage of the disease). For this purpose, the patients with Alzheimer's disease but without psychotic symptoms were considered as control and compared to patients with psychotic symptoms. RESULTS: Nearly half the patients had psychotic symptoms. Delusions appeared earlier and were more frequent than hallucinations and misinterpretations. The more severe was the cognitive impairment, the more frequent and intense were psychotic symptoms, but the difference was significant only in cases with severe cognitive impairment. Hallucinations appeared mainly in patients with advanced dementia and were related firstly with the intensity of functional and cognitive impairment and secondly with the duration of the disease. CONCLUSIONS: Mild psychotic symptoms, especially delusions, appear early during the course of Alzheimer's disease. The frequency and intensity of these symptoms increase in parallel with the functional and cognitive impairments caused by the disease. Hallucinations, which appear mainly when the dementia is severe, can be considered as an evolutive marker of the process. Psychotic symptoms differ from those occurring in other disorders, either neurologic or psychiatric in nature.